Optic Nerve Glioma: Usually presenting as a gradual, painless, unilateral proptosis, optic nerve gliomas are uncommon in children. 25-50% of cases involve neurofibromatosis patients. Patients present in the first decade with unilateral proptosis with decreased vision and an afferent pupillary defect. Optic atrophy, disk edema, or strabismus may be a part of the presenting exam. On CT scan (1), enlargement of the optic foramen with a fusiform enlargement of the optic nerve is often noted. Sudden increase in proptosis can occur, felt secondary to hydration of the loose stroma of the tumor. Most tumors act as benign hamartomas in children, with only a few extending intracranially. Routine follow-up with imaging studies is recommended. Surgical resection is reserved for progressive or severely disabilitated patients. In contrast, optic nerve gliomas in middle aged adults are rare but usually highly malignant if not associated with neurofibromatosis. Back to Orbit